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The first three well-documented cases of pure, extraskeletal telangiectatic osteosarcoma of the soft tissues are presented in this article. The distinctive gross features were a predominance of large, blood-filled spaces, which, on histologic examination, were large blood-filled cavities in association with cyst-like walls containing anaplastic spindle cells and definite osteoid production in two of three cases. One of the cases is of additional clinical interest because the teen-aged daughter of the woman patient had died 1 year previously of a high-grade intramedullary osteosarcoma of bone.

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Over the past 10 years there has been significant progress made in the recognition and treatment of soft tissue sarcomas. With the advent of CAT scans and MRI, preoperative delineation of soft tissue tumors has become readily available. The diagnostic use of these modalities in patients presenting with an ill-defined asymptomatic mass has been extremely helpful in terms of screening patients to decide whether or not a biopsy is indicated. These techniques have also provided a much clearer delineation of the anatomic extent of the primary tumor, which has been of great assistance both in radiation therapy treatment calculations as well as in preoperative surgical planning. The recognition that tumor grade is the dominant prognostic variable has resulted in the more common use of a grading system, and a more uniform reporting and stratification of end results. Recent studies with immunohistochemical staining have proven of value in determining the histogenesis of many tumors that in the past were difficult to classify accurately. Most recently the use of flow cytometry suggests that this will also be a valuable adjunct in determining tumor grade and thereby prognosis. The most recent investigations of molecular biologic evaluation of genetic DNA and RNA sequences, as well as of oncogenes are extremely interesting from a diagnostic standpoint and in demonstrating the potential of molecular biologic evaluation for understanding the origin of these tumors. Multimodality therapy with surgery, radiation, chemotherapy, or all three has resulted in a marked improvement in local tumor control for patients with soft tissue sarcomas. The combination of modalities has allowed smaller surgical excisions of the tumor and thereby preservation of the extremity and much of its function. There are currently several different methods of multimodality therapy used including neoadjuvant therapy and postoperative therapy, both of which have been proven efficacious. Chemotherapy is now playing an increased role in clinical investigation and treatment. The availability of Adriamycin, DTIC, cisplatin, and most recently ifosfamide has added several chemotherapeutic agents for use by the clinician. Combination chemotherapy and radiation is of value in the neoadjuvant setting, and several studies are now underway to determine whether postoperative adjuvant chemotherapy is of similar value in reducing systemic spread of disease. Finally, surgical resection of pulmonary metastases has been proven of value in 20% to 25% of patients who subsequently develop metastatic disease. As a result of these advances in several different treatment disciplines, the overall survival rate and quality of life of patients with soft tissue sarcoma have improved markedly over the past 10 years.(ABSTRACT TRUNCATED AT 400 WORDS)

The first three well-documented cases of pure, extraskeletal telangiectatic osteosarcoma of the soft tissues are presented in this article. The distinctive gross features were a predominance of large, blood-filled spaces, which, on histologic examination, were large blood-filled cavities in association with cyst-like walls containing anaplastic spindle cells and definite osteoid production in two of three cases. One of the cases is of additional clinical interest because the teen-aged daughter of the woman patient had died 1 year previously of a high-grade intramedullary osteosarcoma of bone.

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Over the past 10 years there has been significant progress made in the recognition and treatment of soft tissue sarcomas. With the advent of CAT scans and MRI, preoperative delineation of soft tissue tumors has become readily available. The diagnostic use of these modalities in patients presenting with an ill-defined asymptomatic mass has been extremely helpful in terms of screening patients to decide whether or not a biopsy is indicated. These techniques have also provided a much clearer delineation of the anatomic extent of the primary tumor, which has been of great assistance both in radiation therapy treatment calculations as well as in preoperative surgical planning. The recognition that tumor grade is the dominant prognostic variable has resulted in the more common use of a grading system, and a more uniform reporting and stratification of end results. Recent studies with immunohistochemical staining have proven of value in determining the histogenesis of many tumors that in the past were difficult to classify accurately. Most recently the use of flow cytometry suggests that this will also be a valuable adjunct in determining tumor grade and thereby prognosis. The most recent investigations of molecular biologic evaluation of genetic DNA and RNA sequences, as well as of oncogenes are extremely interesting from a diagnostic standpoint and in demonstrating the potential of molecular biologic evaluation for understanding the origin of these tumors. Multimodality therapy with surgery, radiation, chemotherapy, or all three has resulted in a marked improvement in local tumor control for patients with soft tissue sarcomas. The combination of modalities has allowed smaller surgical excisions of the tumor and thereby preservation of the extremity and much of its function. There are currently several different methods of multimodality therapy used including neoadjuvant therapy and postoperative therapy, both of which have been proven efficacious. Chemotherapy is now playing an increased role in clinical investigation and treatment. The availability of Adriamycin, DTIC, cisplatin, and most recently ifosfamide has added several chemotherapeutic agents for use by the clinician. Combination chemotherapy and radiation is of value in the neoadjuvant setting, and several studies are now underway to determine whether postoperative adjuvant chemotherapy is of similar value in reducing systemic spread of disease. Finally, surgical resection of pulmonary metastases has been proven of value in 20% to 25% of patients who subsequently develop metastatic disease. As a result of these advances in several different treatment disciplines, the overall survival rate and quality of life of patients with soft tissue sarcoma have improved markedly over the past 10 years.(ABSTRACT TRUNCATED AT 400 WORDS)

A patient with homozygous-S sickle-cell disease complicated by malignant (fibrous) histiocytoma arising within a tibial medullary infarct is reported. This is the ninth reported case of human sarcoma associated with bone infarct and the first associated with sickle-cell disease. Evidence for a causal relationship is presented.

Primary hemangiopericytoma of bone is a rare malignant tumor. Forty-one cases from the literature were reviewed, and four additional cases are reported. The peak incidence is in fourth and fifth decades. The pelvis and lower extremities are the most common locations. The radiographic features are nonspecific, although angiography may aid in the diagnosis. Based on a limited number of evaluable cases, the prognosis seems to correlate with the histologic grade of the tumor. Late recurrence and distant metastases are common.

In three cases of oligodendrogliomas, atypical cells rich in eosinophilic cytoplasm, resembling plump astroyctic or astroblastic cells, were studied under the electron microscopy. These cells showed finely granular cytoplasm with routine hematoxylin-eosin preparation; ultrastructurally, they exhibited abundant, round cytoplasmic bodies of autophagic-vacuole type, in addition to other fine structures characteristic of oligodendroglioma cells.

A unique case of an elastofibroma arising in the region of the deltoid muscle in a 53-year-old athletic male physician is reported. The lesion was characterized by abundant benign fibrous tissue and sparse accumulations of elastic fibers. The staining of the elastic fibers was accomplished by the use of special staining techniques and are compared to those of other elastofibromas. A review of the literature showed that 56 cases of elastofibroma have been reported to date. Only 2 of these were in an extrascapular site; in 6 patients the lesions were bilateral. The elastofibroma is a completely benign lesion which never recurs after excision. A discussion of the possible pathogenesis of this lesion based on observations of one of the authors (O.H.J.) and his colleagues is also presented. It is suggested that the elastofibroma is a degenerative pseudotumor resulting from local factors such as increased mechanical stress and/or nutritional derangement consequent to vascular involvement.

Although the American Joint Commission has classified all synovial sarcomas as "high grade," histologic subtypes can be identified. By histologically subclassifying synovial sarcoma tumors according to percent glandularity and mitotic rates, the authors were able to define high-risk and low-risk patients. Charts and original pathologic slides were reviewed on 45 synovial sarcoma patients. With a 41-month median follow-up, the low-risk patients showed 100% survival, whereas the high-risk patients showed 37% survival.

A case is presented which is interpreted as a histologically pseudomalignant osteoblastoma. This diagnosis is supported by the clinical and radiologic findings. Arguments are presented in support of this diagnosis as against an osteogenic sarcoma with an arrest in growth, although this latter diagnosis cannot be completely excluded.

A case is presented which is interpreted as a histologically pseudomalignant osteoblastoma. This diagnosis is supported by the clinical and radiologic findings. Arguments are presented in support of this diagnosis as against an osteogenic sarcoma with an arrest in growth, although this latter diagnosis cannot be completely excluded.

A patient with homozygous-S sickle-cell disease complicated by malignant (fibrous) histiocytoma arising within a tibial medullary infarct is reported. This is the ninth reported case of human sarcoma associated with bone infarct and the first associated with sickle-cell disease. Evidence for a causal relationship is presented.

Although the American Joint Commission has classified all synovial sarcomas as "high grade," histologic subtypes can be identified. By histologically subclassifying synovial sarcoma tumors according to percent glandularity and mitotic rates, the authors were able to define high-risk and low-risk patients. Charts and original pathologic slides were reviewed on 45 synovial sarcoma patients. With a 41-month median follow-up, the low-risk patients showed 100% survival, whereas the high-risk patients showed 37% survival.

Primary hemangiopericytoma of bone is a rare malignant tumor. Forty-one cases from the literature were reviewed, and four additional cases are reported. The peak incidence is in fourth and fifth decades. The pelvis and lower extremities are the most common locations. The radiographic features are nonspecific, although angiography may aid in the diagnosis. Based on a limited number of evaluable cases, the prognosis seems to correlate with the histologic grade of the tumor. Late recurrence and distant metastases are common.

Case histories of 10 patients with neurofibrosarcoma, including 14 (70%) with neurofibromatosis, evaluated over 10 years were reviewed to determine the incidence of local and systemic recurrence and the most effective means of therapy for this rare neoplasm. Initial therapy resulted in complete local disease control in only 11 (55%) patients. Local excision, or local excision plus radiation or chemotherapy resulted in local recurrence in 8 of 12 patients. Radical surgery alone, or radical surgery combined with radiation and chemotherapy resulted in local recurrence in 1 of 6. Even with complete local disease control, 7 of 16 (44%) patients died of metastases. Both A.J.C. Clinical Stage II and III patients had a similar poor prognosis. Associated neurofibromatosis did not worsen prognosis. These data suggest that both moderate and high-grade primary neurofibrosarcoma are highly malignant neoplasms and should be treated by radical resection. Preoperative intraarterial Adriamycin and radiation--found to be successful for other highly malignant sarcomas--may be of benefit. Since distant disease occurs despite local control, postoperative adjuvant chemotherapy trials are warranted.

A case of alveolar soft part sarcoma arising some 20 years later in a site previously irradiated as a treatment for a spinal cord hemangioma is described. This is the first known case of radiation-associated alveolar soft part sarcoma, and it fulfills the criteria for a tumor to be radiation-induced. The coincidental finding of ''viral-like'' particles within some of the tumor cells was noted.

In three cases of oligodendrogliomas, atypical cells rich in eosinophilic cytoplasm, resembling plump astroyctic or astroblastic cells, were studied under the electron microscopy. These cells showed finely granular cytoplasm with routine hematoxylin-eosin preparation; ultrastructurally, they exhibited abundant, round cytoplasmic bodies of autophagic-vacuole type, in addition to other fine structures characteristic of oligodendroglioma cells.